{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T03:10:58Z","timestamp":1790997058836,"version":"4.1.0"},"reference-count":42,"publisher":"European Respiratory Society (ERS)","issue":"2","license":[{"start":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T00:00:00Z","timestamp":1774483200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/publications.ersnet.org\/user-licence"}],"funder":[{"name":"Programme Hospitalier de Recherche Clinique","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2026,3,11]]},"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Pulmonary hypertension (PH) frequently complicates interstitial lung diseases (ILDs), adversely affecting outcome. Identifying prognostic factors of patients with PH associated with ILD (ILD-PH) could facilitate early identification of patients who may benefit from PH therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We included patients with ILD-PH from the prospective HYPID cohort and the French National Pulmonary Hypertension Registry (2007\u20132022). Univariable and multivariable analyses were performed to identify predictors of 1-year mortality.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 581 patients (mean age 69.4\u00b19.3\u2005years; 450 males) were analysed. ILD diagnoses were combined pulmonary fibrosis and emphysema (CPFE) syndrome (30.8%), idiopathic pulmonary fibrosis (29.6%), unclassifiable ILD (13.1%) and fibrotic hypersensitivity pneumonitis (10.3%). Mean pulmonary arterial pressure was 40.7\u00b19.1\u2005mmHg and mean pulmonary vascular resistance (PVR) was 7.6\u00b13.5 Wood units (WU). Off-label PH therapy was initiated after initial evaluation in 215 patients (37%). The median transplant-free survival time was 17 (95% CI 15.2\u2013not reached)\u2005months. Multivariable analysis identified male sex (p&lt;0.001), World Health Organization functional class (WHO FC) III (p=0.003) or IV (p=0.003), 6-min walk distance (6MWD) \u2264228\u2005m (p&lt;0.001), PVR &gt;5\u2005WU (p=0.008) and absence of PH therapy (p&lt;0.001) as independent predictors of death or lung transplantation at 1\u2005year.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Non-invasive (6MWD and WHO FC) and invasive (PVR) variables are associated with prognosis in patients with ILD-PH, including in patients with CPFE. PH medication might improve outcomes in this patient population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1183\/13993003.02233-2025","type":"journal-article","created":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T17:20:17Z","timestamp":1774545617000},"page":"2502233","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":6,"title":["Prognostic factors in interstitial lung disease-associated pulmonary hypertension: data from the HYPID cohort and the French Pulmonary Hypertension Registry"],"prefix":"10.1183","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0746-357X","authenticated-orcid":false,"given":"R\u00e9mi","family":"Diesler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9gol\u00e8ne","family":"Turquier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Reynaud-Gaubert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Lestelle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Lacoste-Palasset","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4870-3923","authenticated-orcid":false,"given":"Victor","family":"Valentin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9bastien","family":"Qu\u00e9tant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6056-202X","authenticated-orcid":false,"given":"Ari","family":"Chaouat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cl\u00e9ment","family":"Boissin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c9lise","family":"No\u00ebl-Savina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9cile","family":"Tromeur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lien","family":"Justet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnaud","family":"Maurac","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c9lise","family":"Artaud-Macari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hilario","family":"Nunes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8214-3010","authenticated-orcid":false,"given":"Laurent","family":"Bertoletti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Magro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Horeau-Langlard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-France","family":"Seronde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Favrolt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romain","family":"Tr\u00e9sorier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bouchra","family":"Lamia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Chabanne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"Riou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9bastien","family":"Renard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Sanchez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4231-5102","authenticated-orcid":false,"given":"Fr\u00e9deric","family":"Gagnadoux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Nieves","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kais","family":"Ahmad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Traclet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1942-1951","authenticated-orcid":false,"given":"Olivier","family":"Sitbon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Lamblin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1644-7264","authenticated-orcid":false,"given":"Bruno","family":"Degano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9304-1359","authenticated-orcid":false,"given":"Simon","family":"Valentin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4645-5209","authenticated-orcid":false,"given":"Arnaud","family":"Bourdin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0703-2892","authenticated-orcid":false,"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6246-5557","authenticated-orcid":false,"given":"Ath\u00e9na\u00efs","family":"Boucly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabien","family":"Subtil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-0955","authenticated-orcid":false,"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the PulmoTension and OrphaLung networks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2026,3,26]]},"reference":[{"key":"2026080610552177000_68.2.2502233.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(22)01052-2"},{"key":"2026080610552177000_68.2.2502233.2","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1016\/j.chest.2024.04.025","article-title":"Pulmonary hypertension in interstitial lung disease: a systematic review and meta-analysis","volume":"166","author":"Ang","year":"2024","journal-title":"Chest"},{"key":"2026080610552177000_68.2.2502233.3","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01324-2024"},{"key":"2026080610552177000_68.2.2502233.4","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1159\/000524263","article-title":"The effect of borderline pulmonary hypertension on survival in chronic lung disease","volume":"101","author":"Piccari","year":"2022","journal-title":"Respiration"},{"key":"2026080610552177000_68.2.2502233.5","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2017.02.016"},{"key":"2026080610552177000_68.2.2502233.6","doi-asserted-by":"crossref","first-page":"e0141911","DOI":"10.1371\/journal.pone.0141911","article-title":"Pulmonary hypertension in patients with chronic fibrosing idiopathic interstitial pneumonias","volume":"10","author":"Hoeper","year":"2015","journal-title":"PLoS One"},{"key":"2026080610552177000_68.2.2502233.7","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1111\/resp.14378","article-title":"Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: a Bayesian retrospective observational cohort study","volume":"28","author":"Dawes","year":"2023","journal-title":"Respirology"},{"key":"2026080610552177000_68.2.2502233.8","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1164\/rccm.202412-2371OC","article-title":"Hemodynamics and phosphodiesterase-5 inhibitor treatment associated with survival in ILD-PH: a PVRI GoDeep meta-registry analysis","volume":"211","author":"Yogeswaran","year":"2025","journal-title":"Am J Respir Crit Care Med"},{"key":"2026080610552177000_68.2.2502233.9","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2008470"},{"key":"2026080610552177000_68.2.2502233.10","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02414-2022"},{"key":"2026080610552177000_68.2.2502233.11","first-page":"100948","article-title":"French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis \u2013 2021 update. Full-length version","volume":"83","author":"Cottin","year":"2023","journal-title":"Respir Med Res"},{"key":"2026080610552177000_68.2.2502233.12","doi-asserted-by":"publisher","DOI":"10.1016\/0091-6749(89)90349-7"},{"key":"2026080610552177000_68.2.2502233.13","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2009-040GL"},{"key":"2026080610552177000_68.2.2502233.14","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201807-1255ST"},{"key":"2026080610552177000_68.2.2502233.15","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202005-2032ST"},{"key":"2026080610552177000_68.2.2502233.16","doi-asserted-by":"crossref","first-page":"e7","DOI":"10.1164\/rccm.202206-1041ST","article-title":"Syndrome of combined pulmonary fibrosis and emphysema: an official ATS\/ERS\/JRS\/ALAT research statement","volume":"206","author":"Cottin","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"2026080610552177000_68.2.2502233.17","doi-asserted-by":"publisher","DOI":"10.1002\/art.30077"},{"key":"2026080610552177000_68.2.2502233.18","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00021005"},{"key":"2026080610552177000_68.2.2502233.19","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00038709"},{"key":"2026080610552177000_68.2.2502233.20","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00197-2024"},{"key":"2026080610552177000_68.2.2502233.21","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00889-2017"},{"key":"2026080610552177000_68.2.2502233.22","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00740-2017"},{"key":"2026080610552177000_68.2.2502233.23","doi-asserted-by":"crossref","first-page":"2102311","DOI":"10.1183\/13993003.02311-2021","article-title":"COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension","volume":"60","author":"Hoeper","year":"2022","journal-title":"Eur Respir J"},{"key":"2026080610552177000_68.2.2502233.24","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1016\/j.chest.2020.08.2069","article-title":"Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension","volume":"159","author":"Benza","year":"2021","journal-title":"Chest"},{"key":"2026080610552177000_68.2.2502233.25","doi-asserted-by":"crossref","first-page":"100206","DOI":"10.1016\/j.jhlto.2025.100206","article-title":"Derivation of a simple risk calculator for predicting clinical worsening in patients with pulmonary hypertension due to interstitial lung disease","volume":"7","author":"El-Kersh","year":"2025","journal-title":"JHLT Open"},{"key":"2026080610552177000_68.2.2502233.26","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/j.healun.2022.03.011","article-title":"Prognostic value of improvement endpoints in pulmonary arterial hypertension trials: a COMPERA analysis","volume":"41","author":"Hoeper","year":"2022","journal-title":"J Heart Lung Transplant"},{"key":"2026080610552177000_68.2.2502233.27","doi-asserted-by":"crossref","first-page":"e0193226","DOI":"10.1371\/journal.pone.0193226","article-title":"Association between six-minute walk distance and long-term outcomes in patients with pulmonary arterial hypertension: data from the randomized SERAPHIN trial","volume":"13","author":"Souza","year":"2018","journal-title":"PLoS One"},{"key":"2026080610552177000_68.2.2502233.28","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.chest.2018.01.009","article-title":"Baseline and serial brain natriuretic peptide level predicts 5-year overall survival in patients with pulmonary arterial hypertension: data from the REVEAL registry","volume":"154","author":"Frantz","year":"2018","journal-title":"Chest"},{"key":"2026080610552177000_68.2.2502233.29","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01323-2024"},{"key":"2026080610552177000_68.2.2502233.30","doi-asserted-by":"publisher","DOI":"10.1136\/openhrt-2022-002054"},{"key":"2026080610552177000_68.2.2502233.31","doi-asserted-by":"publisher","DOI":"10.1136\/thorax-2023-220179"},{"key":"2026080610552177000_68.2.2502233.32","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01483-2021"},{"key":"2026080610552177000_68.2.2502233.33","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2008.112847"},{"key":"2026080610552177000_68.2.2502233.34","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01537-2014"},{"key":"2026080610552177000_68.2.2502233.35","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01638-2025"},{"key":"2026080610552177000_68.2.2502233.36","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022"},{"key":"2026080610552177000_68.2.2502233.37","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01200-2024"},{"key":"2026080610552177000_68.2.2502233.38","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp-2023-002116"},{"key":"2026080610552177000_68.2.2502233.39","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1111\/resp.12530","article-title":"Multi-institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases","volume":"20","author":"Tanabe","year":"2015","journal-title":"Respirology"},{"key":"2026080610552177000_68.2.2502233.40","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/j.jacasi.2024.01.009","article-title":"Pulmonary hypertension with interstitial pneumonia: initial treatment effectiveness and severity in a Japan registry","volume":"4","author":"Tanabe","year":"2024","journal-title":"JACC Asia"},{"key":"2026080610552177000_68.2.2502233.41","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/art.41512","article-title":"Hemodynamic response to treatment and outcomes in pulmonary hypertension associated with interstitial lung disease versus pulmonary arterial hypertension in systemic sclerosis: data from a study identifying prognostic factors in pulmonary hypertension associated with interstitial lung disease","volume":"73","author":"Chauvelot","year":"2021","journal-title":"Arthritis Rheumatol"},{"key":"2026080610552177000_68.2.2502233.42","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1111\/resp.13250","article-title":"Pulmonary hypertension in interstitial lung disease: limitations of echocardiography compared to cardiac catheterization","volume":"23","author":"Keir","year":"2018","journal-title":"Respirology"}],"container-title":["European Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/13993003.02233-2025","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T17:55:32Z","timestamp":1786038932000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/13993003.02233-2025"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,26]]},"references-count":42,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,8,6]]},"published-print":{"date-parts":[[2026,8]]}},"alternative-id":["10.1183\/13993003.02233-2025"],"URL":"https:\/\/doi.org\/10.1183\/13993003.02233-2025","relation":{},"ISSN":["0903-1936","1399-3003"],"issn-type":[{"value":"0903-1936","type":"print"},{"value":"1399-3003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,26]]}}}